Primary chiasmal germinoma. A case report and review of the literature

Abstract

A case is presented in which an adult man with painless progressive loss of vision subsequently was found to have a primary suprasellar/perichiasmal germinoma (ectopic pinealoma). A review of the literature revealed 93 similar cases of germinoma occurring in the perichiasmal region and these are tabulated. The diagnosis and management of this lesion are discussed, including the recognition of the characteristic neuroendocrinologic triad of diabetes insipidus, visual changes, and hypopituitarism. Of 64 cases from the literature wherein presenting symptoms were reported, 56 (87.5%) had diabetes insipidus, 53 (82.8%) visual changes, and 36 (56%) hypopituitarism. A review of the literature suggests that diabetes insipidus is usually the initial symptom in suprasellar germinoma. However, we wish to emphasize the ophthalmologic presentation of this entity, because of ophthalmologic presentation of this entity, because patients in the age group most affected (adolescents) will often not recognize symptoms of diabetes insipidus, but will first seek medical attention for painless progressive loss of vision suggestive of chiasmal compression. The radiosensitivity of this lesion is also discussed. Of 61 patients receiving irradiation therapy, 42 (68%) were surviving at the time of their individual case report. No patient in the review survived without irradiation therapy.